Alternations in genes expression of pathway signaling in esophageal tissue with atresia: results of expression microarray profiling.

Esophageal atresia (EA) is a congenital defect of the esophagus involving the interruption of the esophagus with or without connection to the trachea (tracheoesophageal fistula [TEF]). EA/TEF may occur as an isolated anomaly, may be part of a complex of congenital defects (syndromic), or may develop within the context of a known syndrome or association. The molecular mechanisms underlying the development of EA are poorly understood. It is supposed that a combination of multigenic factors and epigenetic modification of genes play a role in its etiology. The aim of our work was to assess the human gene expression microarray study in esophageal tissue samples. Total RNA was extracted from 26 lower pouches of esophageal tissue collected during thoracoscopic EA repair in neonates with the isolated (IEA) and the syndromic form (SEA). We identified 787 downregulated and 841 upregulated transcripts between SEA and controls, and about 817 downregulated and 765 upregulated probes between IEA and controls. Fifty percent of these genes showed differential expression specific for either IEA or SEA. Functional pathway analysis revealed substantial enrichment for Wnt and Sonic hedgehog, as well as cytokine and chemokine signaling pathways. Moreover, we performed reverse transcription polymerase chain reaction study in a group of SHH and Wnt pathways genes with differential expression in microarray profiling to confirm the microarray expression results. We verified the altered expression in SFRP2 gene from the Wnt pathway as well as SHH, GLI1, GLI2, and GLI3 from the Sonic hedgehog pathway. The results suggest an important role of these pathways and genes for EA/TEF etiology.

Ten years later-scalp still a primary donor site in children.

UNLABELLED: Choice of the donor site for a split thickness skin graft depends on skin availability, possible complications and anticipated esthetic results. We selected the scalp to be the primary donor site at our institution. During a period of ten years (1998-2008), a group of 123 pediatric patients aged 4 months to 15 years (65% were below the age of 2; mean age 2.98 years) underwent skin grafting from this particular site. In 2 cases the same area was re-harvested. All donor sites healed by the 10th post-operative day. Donor site complications included: 2 microalopecia regions, 5 pressure sores in a close proximity, 1 hypertrophic scar and 1 visible mark on the forehead due to technical mistake in graft harvesting. All children started scar management of the recipient site with contact therapy using adhesive tape Hypafix (BSN Medical); subsequently moving on to silicone sheets or gel in selected refractory cases. We present results for 68 scars in 41 patients with the longest follow-up period. Scar quality was evaluated after minimum of 10 years and scored according to the Vancouver Scar Assessment Scale. Very good and good results were obtained in 55 scars (80.9%), satisfactory in 11 scars (16.2%) and unsatisfactory in 2 scars (2.9%). CONCLUSIONS: Our results confirm, that the scalp is a reliable donor site in children and contact therapy is an adequate form of scar prevention/treatment of the recipient site.

Paediatric and adolescent traumatic gastrointestinal injuries: results of a European multicentre analysis.

AIM: Paediatric gastrointestinal injuries (GIIs) are rare, and the aim of this multicentre study was to evaluate their outcomes in a large cohort. METHODS: Hospital databases of 10 European paediatric surgical centres were reviewed for paediatric traumatic GIIs managed between 2000-2010. RESULTS: Ninety-seven patients with a median age of 9 years (0-17 years) were identified, with 72 blunt and 25 penetrating GIIs. Initial diagnostics in 90 patients led to correct diagnosis in 71%. Diagnostics were delayed in 26 patients (median 24 h). Eighty-two patients required surgery (67 laparotomy, 12 laparoscopy and three other approaches). There was a 50% conversion in the laparoscopic group. Median hospital stay was 10 days (range 1-137 days), with longer duration influenced by associated injuries (n = 41). Diagnosis <24 h was associated with significantly shorter hospital stay compared to more than 24 h (p = 0.011). In one-third of patients, morbidities were not related to a diagnostic delay or type of injury. There were five lethal outcomes, four due to associated injuries. CONCLUSION: Initial diagnostics in traumatic paediatric GIIs provide false negatives in one-third of patients. Diagnostic delay <24 h is associated with a significantly shorter hospital stay. Although laparoscopy is associated with a conversion rate of 50%, it can be used for diagnosis in suspected cases to avoid nontherapeutic laparotomy.

Fibrolamellar variant of hepatocellular carcinoma does not have a better survival than conventional hepatocellular carcinoma--results and treatment recommendations from the Childhood Liver Tumour Strategy Group (SIOPEL) experience.

PURPOSE: Fibrolamellar hepatocellular carcinoma (FL-HCC) and conventional hepatocellular carcinoma (HCC) cases in two consecutive paediatric HCC trials were analysed to compare outcome and derive treatment implications. PATIENTS AND METHODS: Data of 24 FL-HCC (24% PRETEXT IV) and 38 HCC (42% PRETEXT IV) cases from SIOPEL-2 and -3 (1995-1998, 1998-2006) were analysed. Patients were treated according to SIOPEL-2 and -3 high-risk protocol (carboplatin+doxorubicin alternating with cisplatin; seven preoperative, three postoperative cycles) or with primary surgery followed by chemotherapy as indicated. RESULTS: Thirteen of 24 FL-HCC (54%) and 32/38 HCC (84%) were initially treated with chemotherapy. Eight FL-HCC (33%) and five HCC patients (13%) had primary surgery. Partial response was observed in 31% of FL-HCC versus 53% of HCC patients (p=0.17). Complete resection was achieved in ten FL-HCC and seven HCC patients (p=0.08). Three-year event free survival (EFS) was 22% for FL-HCC versus 28% for HCC. Overall survival (OS) was not significantly different at 3 years follow up (42% for FL-HCC versus 33% for HCC, p=0.24). EFS/OS Kaplan-Meier curves did not differ significantly, with median follow up of 43 (FL-HCC) and 60 (HCC) months. No significant correlation was found between potential prognostic factors and OS. In the entire cohort nine out of 23 (39%) patients with complete resection or orthotopic liver transplantation versus 34/39 (87%) without successful surgical treatment, died. CONCLUSIONS: Long-term OS in FL-HCC and HCC is similar. With low response rates, complete resection remains the treatment of choice.

Relapses in hepatoblastoma patients: clinical characteristics and outcome--experience of the International Childhood Liver Tumour Strategy Group (SIOPEL).

PURPOSE: To analyse the clinical characteristics and outcome of hepatoblastoma (HB) patients who relapsed after enrolment on SIOPEL studies 1-3. PATIENTS AND METHODS: Analysis of clinical data of all 59 patients (pts) registered in SIOPEL 1-3 studies, who relapsed after achieving complete remission (CR). RESULTS: The median time from the initial diagnosis to relapse was 12 months (4-115 m). The site of relapse was lung N=27, liver N=21, both liver and lung N=5 and other N=5 (missing data-MD: 1 patient). All but 9 pts had an alpha-fetoprotein level >10 ng/mL at the time of relapse. Treatment of the relapse included chemotherapy and surgery N=25, chemotherapy alone N=21, surgery alone N=7 and only palliative treatment N=5 (MD: 1 pt). Overall, 31 pts (52%) achieved a second CR. With a median follow-up of 83 months, 23 pts are alive, (18 in 2nd CR, 5 after a second relapse) and 36 pts have died (35 from disease and 1 from complications). Three-year event-free survival and overall survival are 34% and 43% respectively (95% confidence interval [CI] 0.28-0.69). The main factors associated with a good outcome were PRETEXT group I-III at diagnosis, a high AFP level at relapse and relapse treatment including both chemotherapy and surgery. CONCLUSION: Relapses in HB are rare events occurring in less than 12% of pts after CR. Combined treatment with chemotherapy and surgical removal of the tumour is essential for long-term survival.

The state of research into children with cancer across Europe: new policies for a new decade.

Overcoming childhood cancers is critically dependent on the state of research. Understanding how, with whom and what the research community is doing with childhood cancers is essential for ensuring the evidence-based policies at national and European level to support children, their families and researchers. As part of the European Union funded EUROCANCERCOMS project to study and integrate cancer communications across Europe, we have carried out new research into the state of research in childhood cancers. We are very grateful for all the support we have received from colleagues in the European paediatric oncology community, and in particular from Edel Fitzgerald and Samira Essiaf from the SIOP Europe office. This report and the evidence-based policies that arise from it come at a important junction for Europe and its Member States. They provide a timely reminder that research into childhood cancers is critical and needs sustainable long-term support.

Nonparasitic splenic cysts in children: a multicentric study.

PURPOSE: Nonparasitic splenic cysts (NPSCs) are uncommon in children. The aim of this multinational and multicentric study was to present the authors' experience as well as the changing trends in the management of NPSCs over the last 25 years. MATERIAL AND METHODS: From 1981 to 2005, 50 children or adolescents were surgically treated for NPSCs in 6 paediatric surgical centres in four European countries. The medical records of these 50 patients with NPSCs were reviewed retrospectively. RESULTS: Twenty-six male and 24 female patients were operated on. Age at surgery ranged from 1 to 17 years (mean 11.9). Seventeen patients were symptomatic. Six total (4 open and 2 laparoscopic) and 26 partial (22 open and 4 laparoscopic) splenectomies were performed. Laparoscopic fenestration or deroofing and open cystectomy was carried out in 9 patients, respectively. Histological findings revealed the lesion to be an epidermoid cyst (n = 28), a pseudocyst (n = 15) or a mesothelial cyst (n = 2). In 5 patients haemangioma or lymphangioma was the pathological diagnosis. At a mean follow-up of 2.9 years, residual cysts were found in 8 laparoscopically treated patients, 4 of whom required re-do laparoscopy or open surgery. CONCLUSIONS: Over the last two decades, the surgical treatment of NPSCs has changed from a formerly customary total splenectomy to spleen-conserving procedures, such as total cystectomy with or without partial splenectomy or partial cystectomy. These therapeutic modalities can be performed laparoscopically, if technically possible. Fenestration or deroofing of the cyst resulted in a high recurrence rate (7/9).

Risk-adapted treatment for childhood hepatoblastoma. final report of the second study of the International Society of Paediatric Oncology--SIOPEL 2.

SIOPEL 2 was a pilot study designed to test the efficacy and toxicity of two chemotherapy (CT) regimens, one for patients with hepatoblastoma (HB) confined to the liver and involving no more than three hepatic sectors ('standard-risk (SR) HB'), and one for those with HB extending into all four sectors and/or with lung metastases or intra-abdominal extra hepatic spread 'high-risk (HR) HB'. SR-HB patients were treated with four courses of cisplatin (CDDP), at a dose of 80 mg/m(2) every 14 days, delayed surgery, and then two more similar CDDP courses. HR-HB patients were given CDDP alternating every 14 days with carboplatin (CARBO), 500 mg/m(2), and doxorubicin (DOXO), 60 mg/m(2). Two courses of CARBO/DOXO and one of CDDP were given postoperatively. Between October 1995 and May 1998, 77 SR-HB (10 of whom were actually treated with the HR protocol) and 58 HR-HB patients were registered and all 135 could be evaluated. Response rates for the entire SR-HB and HR-HB groups were 90% (95% CI 80-96%) and 78% (95% CI 65-87%), and resection rates were 97% (95% CI 87-99%) and 67% (95% CI 54-79%) including several children undergoing liver transplantation. For SR-HB patients, 3-year overall and progression-free survivals were 91% (+/-7%) and 89% (+/-7%) and for the HR-HB group 53% (+/-13%) and 48% (+/-13%), respectively. The short-term toxicity of these regimens was acceptable, with no toxic deaths. A treatment strategy based on CDDP monotherapy and surgery thus appears effective in SR-HB but, despite CT intensification, only half of the HR-HB patients are long-term survivors. For SR-HB patients, the efficacy of CDDP monotherapy and the CDDP/DOXO ('PLADO') combination are now being compared in a prospective randomised trial (SIOPEL 3).

Liver transplantation for hepatoblastoma: results from the International Society of Pediatric Oncology (SIOP) study SIOPEL-1 and review of the world experience.

BACKGROUND: For hepatoblastoma (HB) that remains unresectable by partial hepatectomy after chemotherapy, total hepatectomy with orthotopic liver transplantation (LTX) has been advocated as the best treatment option. The role of LTX in the overall management of HB is still, however, unclear. PROCEDURE: The results of LTX from the first study of HB by the International Society of Pediatric Oncology, SIOPEL-1, were analyzed. In addition, the world experience of LTX for HB was extensively reviewed. Twelve patients in the SIOPEL-1 study underwent a LTX. Median (range) follow-up at Dec. 31, 2001 was 117 months (52-125) since LTX. RESULTS: Overall survival at 10 years post-LTX was 85% for the seven children who received a "primary LTX" and 40% for the 5 children who underwent a "rescue LTX" after previous partial hepatectomy. In the world experience (147 cases), the overall survival rate at 6 year post-LTX was 82% for 106 patients who received a "primary LTX" and 30% for 41 patients who underwent a "rescue LTX." Multivariate analysis of patients undergoing primary LTX showed that only macroscopic venous invasion had a significant impact (P-value: 0.045 with a hazard ratio of 2.96) on overall survival. CONCLUSIONS: Orthotopic LTX has added a new dimension to the treatment of HB unresectable by partial hepatectomy. Because of the rarity of the disease and to optimize results, children with extensive HB should be treated in centers with surgical expertise in pediatric major liver resection and LTX, in close collaboration with pediatric oncologists, radiologists, and histopathologists.

Adult type vs. Childhood hepatocellular carcinoma--are they the same or different lesions? Biology, natural history, prognosis, and treatment.

BACKGROUND: Differences in the biology, natural history, and treatment results of hepatocellular carcinoma (HCC) in children and adults were sought based on the literature and experience resulting from SIOPEL 1 trial. PROCEDURE: In the SIOPEL1 study, 40 children with HCC were registered from January 1990 to February 1994. Outcome was analyzed in 39. In most cases, disease was advanced at diagnosis: 31% had metastases and 39% had extrahepatic tumor extension and/or vascular invasion. More than 50% of patients had multifocal tumors; 39% of tumors were associated with hepatic cirrhosis. All, but two patients, received preoperative chemotherapy (PLADO--cisplatin and doxorubicin). Outcome, response to treatment, and prognostic factors were analyzed using the SAS statistical package. RESULTS: Overall survival (OS) at 5 years is 28% and EFS is 17% at median follow-up of 75 months (49-90). Partial response to chemotherapy was observed in 18 of 37 cases evaluated (49%). Complete tumor resection was achieved in 14 of the 39 patients (36%). Twenty (51%) never became operable. Multifocality of the tumor, presence of metastases, and PRETEXT grouping adversely influenced OS. A large number of "de novo" HCC cases, fairly high response rate to preoperative chemotherapy (49%) and 54% survival after complete resection constitute a significant difference in comparison with adult HCC series. CONCLUSIONS: Survival for pediatric HCC patients is below 30%. Radical tumor resection remains the only chance for survival. New multi-center prospective studies in children with HCC are required to better results and to allow further study of differences between adult and pediatric HCC should they exist.

Hepatocellular carcinoma in children: results of the first prospective study of the International Society of Pediatric Oncology group.

PURPOSE: To improve survival and reduce operative morbidity and mortality in children with primary epithelial liver tumors by using preoperative chemotherapy, as well as to collect information on the epidemiology, natural history, and prognostic factors. PATIENTS AND METHODS: Forty children with hepatocellular carcinoma (HCC) were registered onto the Group for Epithelial Liver Tumors International Society of Pediatric Oncology's first study from January 1990 to February 1994. The outcome could be analyzed in 39 of those patients. Disease was often advanced at the time of diagnosis; metastases were identified in 31% of the children and extrahepatic tumor extension, vascular invasion, or both in 39%. Multifocal tumors were common (56%). Thirty-three percent of tumors were associated with hepatic cirrhosis. All but two patients received preoperative chemotherapy (cisplatin and doxorubicin). RESULTS: Partial response was observed in 18 (49%) of 37 patients; there was no response or progression in the remainder. Complete tumor resection was achieved in 14 patients (36%). Twenty patients (51%) never became operable. Overall survival at 5 years was 28%, and event-free survival was 17%. Most deaths resulted from tumor progression (26 of 28). Presence of metastases and pretreatment extent of disease system grouping at diagnosis had an adverse influence on overall survival in multivariate analysis. CONCLUSION: Survival for pediatric HCC patients is significantly inferior to that for children with hepatoblastoma. Complete tumor excision remains the only realistic chance of cure, although it is often prevented by advanced disease. The presence of metastases is the most potent predictor of poor prognosis. A prospective worldwide cooperation in the field of pediatric HCC should be encouraged to look for novel therapeutic concepts.

Multicenter retrospective analysis of various primary pediatric malignant hepatic tumors--management in a series of 47 Polish patients (1985-1995).

Forty-seven children treated in various Polish centers between 1985 and 1995 for primary malignant liver tumors were retrospectively analyzed. Hepatoblastoma (HB) prevailed--it was found in 39 cases. There were 6 hepatocarcinoma (HCC) cases and 2 cases of undifferentiated sarcoma (UDS). In 44% of HB patients the tumor involved both liver lobes. 18% of children with HB presented with pulmonary metastases at diagnosis. Chemotherapy was applied in 92% of cases (preoperatively in 67%). Tumor resection was performed in 56% of HB patients. Overall survival of patients with hepatoblastoma was 43.6%, while it was 50% for hepatocarcinoma and 100% for undifferentiated sarcoma (2 cases only). Mean observation time was 58 months. The hepatoblastoma subgroup, being the largest (83% of all cases), was analyzed separately for prognostic factors. Completeness of tumor excision strongly influenced survival. Involvement of both lobes of the liver and multifocality of the tumor were other adverse prognostic factors.

[Application of Ethyol during chemotherapy in patients with impaired kidney function. Description of three cases]. / Zastosowanie ethyolu u pacjentów z uposledzona funkcja nerek poddawanych chemioterapii. Opis 3 przypadków.

Nephrotoxicity is observed in 30% of patient's treated with chemotherapy schedules based on cisplatin analogues. Ethyol (amiphostin) was used as a protectant of kidney in 3 paediatric patients with neoplastic disease. Two patients suffered from Wilms tumour. One of them, a 5 month old boy presented with agenesis of the right kidney and stage II tumour in the left kidney. Bilateral Wilms tumour was recognised in a 6 year old girl. In both cases glomerular filtration was diminished. The third patient was a girl aged 5 who had isolated relapse of neuroblastoma in the central nervous system. Primary treatment caused nephrotoxic complications such as Fanconi syndrome and diminished glomerular filtration (66 ml/min). In these 3 cases Ethyol was used every lime before analogues of cisplatin were given. Tolerance and the effect of this protectant were good.

Cytogenetics of hepatoblastoma: further characterization of 1q rearrangements by fluorescence in situ hybridization: an international collaborative study.

BACKGROUND: Hepatoblastoma (HBT) is the most common hepatic neoplasm in children. This notwithstanding, little is known about pathogenetic factors, such as genetic abnormalities, of importance for the development and progression of this tumor type. To date, only 33 cytogenetically abnormal HBT have been published, and trisomies for chromosomes 2 and 20 have been shown to be the most frequent aberrations. Recently, unbalanced translocations involving proximal 1q have been described in several HBT, suggesting that a pathogenetically important gene maps to 1q. PROCEDURE: Six primary and one recurrent HBT were cytogenetically analyzed after short-term tissue culture. In addition, fluorescence in situ hybridization (FISH) studies, using locus-specific probes, were performed on three of these pediatric HBT as well as on one previously reported adult HBT. RESULTS: Total or partial trisomy 8, gain of chromosome 20, and structural rearrangements of chromosome 1 were detected in three HBT, and overrepresentation of chromosome 2 material was found in two HBT. The adjacent chromosome bands 1q12 and 1q21 were involved in three translocations, t(1;2), t(1;4), and t(1;11), which were all unbalanced and resulted in gain of 1q material. The previously reported adult HBT displayed 1q deletions with breakpoints at 1q12-21. FISH analyses of the 1q rearrangements revealed that all breakpoints were within the heterochromatic region. CONCLUSIONS: These findings provide further support for the importance of trisomies 2, 8, and 20 and rearrangements of 1q in the development of HBT. Furthermore, the consistent localization of breakpoints within the heterochromatic segment of chromosome 1 suggests that the important pathogenetic consequence of 1q abnormalities is the resulting genomic imbalance rather than a specific gene rearrangement.

Venoocclusive liver disease (VOD) as a complication of Wilms' tumour management in the series of consecutive 206 patients.

UNLABELLED: In 4 years (1993-1996) 206 pts. with nephroblastoma were treated. All children were treated according to SIOP 93-01 protocol. Overall survival was 92%. In 27 cases hepatotoxic events occurred. In 10 cases, venoocclusive liver disease (VOD) was diagnosed. VOD is a syndrome associated with hepatomegaly, sudden weight gain or ascites and jaundice. It results from damage to the endothelium of hepatic venules and necrosis of central hepatocytes with subsequent proliferation of fibrous tissue and occlusion of the central hepatic veins. Dactinomycin is one of the drugs considered responsible for its development. Mean age of VOD patients was 4 yrs, however 3 of them were below 1 yr. In all cases, VOD occurred during postoperative chemotherapy (mean 16 th week of treatment). All patients received dactinomycin and vincristine. Five children with right kidney tumors underwent post-operative abdominal irradiation. Main VOD symptoms were hepatomegaly and ascites (80%). Hypertransaminasaemia, as well as, on ultrasound, gallbladder wall thickening and/or free abdominal fluid were observed. Median VOD duration was 27 days and its course was usually temporary and self-limiting. However, in 2 cases recurrent VOD episodes were noted. All children received supportive treatment only. In 6 cases, VOD resulted in chemotherapy delay or drug reductions, while in 4 others chemotherapy was completed preliminarily. Nevertheless it did not affect patients' outcome overall survival in VOD group was 90%. CONCLUSIONS: Total 5% VOD frequency is similar to other reports. Infants and children receiving abdominal irradiation seem to be at special risk of VOD development.

Experimental model of hepatic venoocclusive disease (VOD) caused by dactinomycin--preliminary report about hepatoprotective effect of amifostine.

UNLABELLED: The purpose of the study was elaboration of the experimental model of hepatic venoocclusive disease (VOD) induced by dactinomycin and investigation of possible hepatoprotective effects of amifostine and heparin individually or in combination with dexamethasone. 198 Wistar strain male rats were used in the trial in two series of experiments. In the first series the experimental model of VOD induced by dactinomycin was elaborated on the group of 18 animals (divided into 3 groups receiving intraperitoneally isotonic salt solution, dactinomycin or nitrosamine). Nitrosamine--a well-known agent causing VOD--was used as positive control. Open biopsies of the liver and blood collections were repeated in order to determine liver enzymes' concentrations. Histopathological examinations demonstrated that dactinomycin caused liver lesion corresponding with VOD picture. Second series of animals was divided into 6 groups receiving the following drugs: I--0.9% NaCl solution, II--dactinomycin (ACT), III--ACT + fraxiparine s.c., IV--ACT + fraxiparine + dexamethasone, V--ACT + amifostine. Five animals from each group were sacrificed on the 3rd and 7th day after each cycle of drug administration. Blood was drawn in order to determine the following: AspAT, AlAT, Falk, GGTP and LDH. Intravital wedge biopsies under anesthesia with the use of inactin were also performed. Liver samples were stained with the use of H&E, p. a. S and Gomory's techniques. We did not find significant differences in liver enzymes' levels between the groups. Pathological changes corresponding with VOD picture of different intensification were found in liver samples from all the rats receiving ACT. Changes became more and more intensive after consecutive cycles. Lesion of central veins' and liver sinusoids' endothelium dominated. Fraxiparine administered individually or in combination with dexamethasone did not prevent the lesion. Administration of amifostine before ACT decreased pathomorphological changes in liver. Dactinomycin caused homogenous subclinical liver lesion corresponding with VOD. It may also occur in children receiving ACT in the course of nephroblastoma's treatment. But probably the changes are too subtle to manifest themselves clinically with exception of patients particularly sensitive (for example after previous radiotherapy). Lack of differences observed in liver enzymes' levels between the groups supports the explanation. Markers of lesion of liver vessels' endothelium should be looked for to make more specific diagnostics of VOD possible. Hepatoprotective properties of amifostine need further studies. CONCLUSIONS: 1. It is possible to create the experimental model of VOD induced by dactinomycin administration. 2. Amifostine seems to act hepatoprotectively to liver lesions caused by dactinomycin.

Association of inflammatory pseudotumor of the liver and Papillon-Lefevre syndrome--case report.

A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process.

Association of Klinefelter syndrome and abdominal teratoma: a case report.

Extragonadal germ cell tumors are rare. The association with Klinefelter syndrome has become observed recently. A case of an 11-month-old infant with Klinefelter syndrome and a retroperitoneal mature teratoma is presented. In the tumor and lymphocytes, a 47,XXY karyotype was found. The association of Klinefelter syndrome with germ cell tumors and its possible explanations are discussed.

Cytogenetic findings in an embryonal sarcoma of the liver.

An undifferentiated embryonal sarcoma (malignant mesenchymoma) of the liver from a 5-year-old girl was found to have near-triploid and near-hexaploid clones with several chromosomal rearrangements. This is the first description of the chromosomal changes in this tumor type.

[Ethyol (amifostine): application trial in pediatric oncology]. / Ethyol (Amifostyna)--próba zastosowania w onkologii pediatrycznej.

UNLABELLED: Myelotoxicity is one of the major chemotherapeutic side effects. In some adult and paediatric studies it has been shown that amifostine protects bone marrow from toxic effects of alkylating agents and platinum compounds without reduction in overall cytotoxic action. AIM OF THE STUDY: To test an efficacy of amifostine as a myeloprotectant in multiagent chemotherapy containing alkylating agents or platinum analogues. Amifostine was used in 8 children from 3 to 15 yrs of age treated with chemotherapy (CHT) for cancers. It made total number of 28 courses. Amifostine was administered every other CHT course in a dose of 750 mg/m2. The degree of myelotoxicity measured at nadir was compared between with- and without amifostine CHT courses. Anaemia, leucopenia and thrombocytopenia of WHO grades I and II were qualified as mild toxicity while grades III and IV were recognized as severe. RESULTS: Severe anaemia, leucopenia and thrombocytopenia were found after 3/14 (21%), 8/14 (57%) and 6/14 (43%) courses with amifostine. Proportion of these side effects in identical CHT courses without amifostine in the same pts. was as followed: 1/14 (7%), 7/14 (50%) and 9/14 (64%). Differences among both groups were statistically significant (p = 0.025). Mild side effects (nausea, vomiting, transient hypotension) accompanied amifostine administration in 29% of courses (4/14). CONCLUSION: Preliminary results suggest that amifostine decreased the number of severe thrombocytopenias after CHT. The drug was well tolerated by children.